ORIGINAL ARTICLE

Pheochromocytoma: 16 Years of Experience in a Single Center
Feokromasitoma: Tek Merkezde 16 Yıllık Deneyim
Received Date : 18 Sep 2020
Accepted Date : 27 Nov 2020
Available Online : 11 Jan 2021
Doi: 10.25179/tjem.2020-79054 - Makale Dili: EN
Turk J Endocrinol Metab. 2021;25:54-67
Bu makale, CC BY-NC-SA altında lisanslanmış açık erişim bir makaledir.
ABSTRACT
Objective: Reviewing the 16-year experience of pheochromocytoma in a tertiary referral center. Material and Methods: The demographics data and the results of clinical, biochemical, and radiological evaluations of 67 patients who received a diagnosis of pheochromocytoma between the years 2004 and 2020 were obtained retrospectively. Results: The mean age (±SD) of the patients at the time of diagnosis was 46 years (±16.1) with a slight female predominance. The percentage of patients diagnosed due to complaints was 50.8%, while 31.2% were diagnosed during the adrenal incidentaloma screening, and 18% were diagnosed during screening for hereditary conditions. Pre-existing hypertension was detected in 56.7% of the patients, while 11.9% of the patients were diagnosed to have hypertension at the time of diagnosis. Paroxysmal pattern was observed in 53.7% of the patients and was accompanied by the classical triad of palpitation (32.8%), headache (20.9%), and sweating (14.9%) as the leading symptoms. Median tumor size was 40 mm (range: 9-90 mm) and the lesion size correlated significantly (p<0.001) with the urinary catecholamine metabolite levels. The overall rate of hemodynamic instability in both perioperative and postoperative periods was 6%. Hereditary syndromes, including multiple endocrine neoplasia type 2A (MEN 2A), MEN 2B, von Hippel-Lindau (VHL), and neurofibromatosis type 1 (NF1), were diagnosed in 24% of these patients. Hereditary pheochromocytomas were diagnosed at younger ages, and bilateral lesions were more prevalent in hereditary pheochromocytomas (p=0.003 and p<0.001, respectively). In addition, patients with hereditary pheochromocytomas were more asymptomatic rather than sporadic (p=0.016). Metastasis was detected in 3% of these patients. Conclusion: Pheochromocytoma is a rare, life-threatening condition, and therefore, it is important to suspect and test for pheochromocytoma in patients with clinical suspicion. In addition, hereditary syndromes associated with pheochromocytomas should be considered while evaluating patients with pheochromocytoma. A life-long annual follow- up is recommended for the detection of recurrent or metastatic disease, and its evaluation, treatment, and follow-up should involve a multidisciplinary approach in experienced centers.
ÖZET
Amaç: Üçüncü basamak bir üniversite hastanesinde, 16 yıllık feokromasitoma deneyiminin gözden geçirilmesi. Gereç ve Yöntemler: Bu araştırma, 2004- 2020 yılları arasında feokromasitoma tanısı almış 67 hastanın değerlendirildiği retrospektif bir çalışma olup, hastaların demografik özellikleri ve klinik, biyokimyasal ve radyolojik değerlendirmelerine ilişkin sonuçlar araştırılmıştır. Bulgular: Araştırmada, feokromositoma tanısı konulan hastaların yaş ortalaması 46 (±16,1), kadın/erkek oranı 1,2, feokromositoma ile ilişkili yakınmalarla başvurup, bu tanının konulduğu hasta oranı ise %50,8’dir. Hastalara konulan tanıların %31,2’si rastlantısal adrenal kitle araştırılması sırasında, %18’i ise genetik geçişli sendromlar araştırılırken gerçekleştirilmiştir. Hastaların %56,7’sinde tanıdan önce de hipertansiyonun bilindiği, %11.9’unda ise hipertansiyonun tanı sırasında belirlendiği görülmüştür. Hastaların %53.7’sinde tansiyon yüksekliğinin ataklar halinde ortaya çıktığı saptanmıştır. Hastalarda ataklara en sık eşlik eden belirtilerin çarpıntı (%32,8), baş ağrısı (%20,9) ve terleme (%14,9) olduğu dikkati çekmektedir. Araştırmada, ortanca tümör boyutu 40 mm (9-90) saptanmış ve tümör boyutu ile idrar katekolamin metabolit düzeyleri arasında anlamlı korelasyon saptanmıştır (p<0,001). Cerrahi uygulanan hastalarda operasyon sırasında ve sonrasındaki takiplerde hemodinamik dengesizlik görülme sıklığı %6 olarak bulunmuştur. Hastaların %24’üne genetik geçişli sendromlar eşlik etmekte olup, bu sendromlar arasında multipl endokrin neoplazi tip 2A (MEN 2A), MEN 2B, von Hippel-Lindau (VHL) ve nörofibromatozis tip 1 (NF1) yer almaktadır. Öte yandan, kalıtsal feokromositomalı hastaların daha erken yaşlarda tanı aldıkları ve bu hastalarda adrenal lezyonların iki taraflı olma eğilimi gösterdikleri belirlenmiştir (p=0,003 ve p<0,001). Ayrıca, kalıtsal feokromositomalı hastaların, sporadik vakalara göre bulgu vermeden tanı aldıkları görülmüştür (p=0,016). Çalışmamızda, feokromositoma tanılı hastalarda metastaz oranının %3 olduğu saptanmıştır. Sonuç: Feokromositoma, ender görülmesine karşın yaşamı tehdit edebilen bir durumdur. Bu nedenle, klinik açıdan kuşku duyulan hastalarda feokromasitoma tanısını akla getirmek ve hastaları bu açıdan irdelemek büyük önem taşımaktadır. Ayrıca bu tanının konulduğu hastalarda altta yatan genetik sendromların da olabileceği özellikle dikkate alınmalıdır. Hastaların ömür boyu yıllık olarak takip edilmesi, nüks ve metastaz belirlemesi açısından ayrı bir önem taşımaktadır. Öte yandan, bu hastalıkla ilgili olarak değerlendirme, tedavi ve takip çalışmalarının, multidisipliner bir yaklaşımla deneyimli merkezlerde yapılmasına ayrı bir özen gösterilmelidir.
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